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Sunday, November 20, 2016

So this will likely be a controversial post since I know a huge number of people in both Lyme and dysautonomia communities have been diagnosed with POTS.........

POTS stands for postural orthostatic tachycardia syndrome. By definition, it is a rise in heart rate of 30 bpm or a sustained heart rate of 120+ upon standing. It can cause a multitude of symptoms, including fainting; however, it's a benign condition. The need for treatment is to improve symptoms, not as a lifesaving measure. There is a TON of advocacy, fundraising and research going on specifically for POTS. It has definitely claimed the spotlight in the dysautonomia realm. One of the main advocacy associations for dysautonomia focuses almost exclusively on POTS. With limited advocacy efforts for other causes of fainting, including other disorders that fall under the dysautonomia umbrella term, many patients are unaware that there could be other more sinister causes behind their fainting episodes.

A few years ago I was diagnosed with POTS. I met criteria. Unfortunately, having that benign explanation/diagnosis on my chart led to a delay in diagnosing and treating another serious condition with similar symptoms that required a pacemaker: heart block. The physical symptoms are very similar to POTS, but since I had already demonstrated on ECG that I had tachycardia, it was difficult to get doctors to believe that I was sporadically changing to the opposite problem; severe bradycardia. Eventually they listened...............after I went into cardiac arrest.

You may very well have POTS, and yes it is benign, but having POTS does not make you immune to developing other conditions and arrhythmia's. It's of vital importance to not settle on a diagnosis too easily when fainting is involved. Make sure before you attribute fainting to POTS (or Lyme), that you have caught your episodes on an ECG. You may need to request a 30 day heart monitor or an implanted loop recorder to do so. Also, do not assume that just treating Lyme is going to resolve everything when it comes to heart symptoms. I know we as Lyme patients are treated horribly by mainstream doctors, but when it comes to heart symptoms, a LLMD is not a specialist and should not be advising or attempting to treat those types of symptoms. Any responsible LLMD will immediately refer a patient with heart symptoms to a cardiologist or electrophysiologist. The latter is a better choice for people whom do not have structural heart disorders (most Lyme and dysautonomia patients fall into that category). Also, remember, you are not going to a cardiologist to treat Lyme or to discuss Lyme. Let your LLMD do that, and let your EP take care of their specialty.........your heart.

I routinely see posts of people who have fainted with warning signs of other disorders saying things like "my POTS is flaring today" (and their heart rate is in the 30's) or "I had that and it will go away with Lyme treatment." These types of statements always scare me. Fainting is nothing to mess around with. Depending on the underlying cause, it can be benign or life threatening, infrequent or constant. If you haven't caught a fainting episode on an ECG, don't assume it's JUST POTS or JUST LYME. There are many other things that could be going on. You could be having vtach, vfib, severe bradycardia, heart block, hypotension, etc.

Some warning signs that your fainting is not just POTS or Lyme are the following:

So I got my first Mediport about 6 months ago. I don't have it for Lyme. I have it due to severe autonomic dysfunction to use for IV saline to support my BP. I have no intrinsic control of hypotension anymore.

A port is a device about the size of a dime (but much thicker) that is implanted under the skin of your chest or sometimes your arm. The outside border of the port is metal and in the center is a silicone circle that can be stuck with a needle and re-seal thousands of times. From the top of the port extends a plastic catheter that enters your jugular vein and extends down into the larger veins near your heart (superior vena cava). Ports allow medications to be delivered intravenously easily. It can also be used for blood draws via a home nurse or at an infusion center. Regular labs will not draw blood off a port.

The specialists involved in implanting ports are either interventional radiologists or general surgeons. The doctor that thinks you need a port sends an order for placement to one of the two. They call you and schedule you for the surgery. Most people get sedation. I do not. Local lidocaine is my preference. Occasionally people also get general anesthesia but that's not routine.

Before you get a port, it's important to arrange how you will care for it. It will need to be locked/flushed with heparin between uses and at least once per month when not in use. The choices at first are an infusion center or home health. Later you can learn to do things yourself. That is my strong preference since the two times someone other than myself touched the port they did not use sterile technique. An infection in a port is a big deal. It can kill you. Your doctor will need to arrange an order to an infusion center or for a home nurse. The doctor will also need to arrange supplies at an infusion pharmacy separately. You will need special needles called huber needles, dressing change kits, end caps, tubing for gravity bags, a pole or a pump, saline flush syringes and heparin flush syringes in addition to whatever meds you will be infusing.

When you arrive for surgery, you'll change into a gown and before doing so, if you are female, they may outline your bra strap in marker to try to avoid placing the port in an irritating spot. Standard questions will be asked and heart, BP, and oxygen monitors will be attached and you. You may get an IV line and pre-operative antibiotics. Then you will be rolled into the procedure room, transferred to a table and draped and sanitized for surgery. You may not meet the doctor doing the surgery in advance. I did not and that created tremendous problems due to my heart condition. When the doctor comes in, he will numb the area with lidocaine shots and make a tiny cut in your upper neck in which to thread the catheter. He'll also make a larger cut down lower and create a pocket for the port to fit into. None of this is severely painful, but it's not pleasant either. It's totally manageable without sedation, but if you are prone to anxiety you might prefer it.

After the catheter is attached to the port and the port is in the pocket they might stitch the port itself onto muscle. This is of benefit because it will be easier to access and less likely to move and flip. They then close the port incision up, access it, inject dye do a quick x-Ray to make sure it's working and then you are all set. Some people have them leave the port accessed. You must have supplies and care organized at home or at an infusion center in advance if you do this. Sometimes they will not let you leave with it accessed. In my case they wouldn't.

I learned quickly that it was going to be safest and most convenient for me to learn how to access the port myself. It's not that difficult. The most important thing is to make sure that you maintain sterile technique. The needle and sterile dressing can stay in for 7 days. During this time you cannot get that area wet. When the needle is not in, you can shower and swim as you wish.

After 5 months, my first port stopped getting blood return. This is a common problem. In my case it had a fibrin sheath confirmed via X-ray. The hospital tried to de-clot it with TPA and it didn't work so I had to get a replacement.

My second port surgery did not go smoothly. I was very unhappy with the specific department I dealt with. They changed providers (new residents) at least 10x over two appointments and every time the plan changed too. There was no communication between them. One of the nurses was not using sterile technique and left me with an unprimed line of air on a pump. The supervising NP refused to communicate with me in advance regarding my pacemaker and she would not allow me to use my magnet or my medication when I needed to for a sustained rate of 160 ventricular paced. She also didn't call my EP or the pacemaker company as she should have. Then she tried to order a med I was allergic to in my IV line. I was in the room with a resident and two RN's only. There was no radiologist supervising, yet several stated that they were "present" when they were not in my notes. So it was billed as if I was with a specialist for hours under their name for a doctor that never once met me. These are the kinds of things most people wouldn't know about since they are usually sedated by that point. I did not have a problem with the resident that did my surgery, but I did have a problem with the overall disorganization and lack of communication in the department. It put me at risk unnecessarily several times. I complained to the "patient relations" office and absolutely nothing was done. I mention this because you, as a reader, deserve reality. Your experience may be great, but it doesn't always go that way. Be prepared for crappy treatment so that you are ready to advocate for yourself. If it goes well, you'll be happily surprised instead of shocked, disappointed and worried about infection like I was.

For information/instructions on how to access a mediport with sterile technique, click here.

Saturday, September 10, 2016

It's been a really really long time since I've written anything for this blog. Sadly, it's not because I'm feeling any better and have moved on or cured myself from Lyme or autonomic dysfunction. The past several months have been a health roller coaster for me. My mentality about the situation has shifted from fighting to try to find the next thing to try that will cure me, to trying to figure out how I'm going to live with these symptoms for the rest of my life through management techniques. I'm at the 5 year point. In many ways I'm no better than the day I was bitten. In some ways (particularly with my autonomic system) I'm a lot worse.

As far as Lyme treatment. I haven't given up, but I'm not sure if my LLMD feels the same way. I'm not feeling like there are too many doctors who have any hope left for me in terms of the resolution of my symptoms. I think we're all starting to think that I may have "reached the limitations of modern medicine" after trying years of herbs, supplmements and antibiotics. I prefer to be on antibiotics to off them. When I was off antibiotics and on herbs only for a year, was when my heart went into the 30's and stopped. I don't want to end up in a situation like Dr. Neil Spector. Eerily, his progression of heart symptoms is remarkably similar to mine. I will stay on antibiotics as long as I can.

For the past year I've been really immersing myself in exercise. I love running. I'm fairly good at it and it's one thing that I haven't wanted to give up despite being chronically ill. With severe hypotension, running sometimes feels next to impossible. My body reacts in the opposite way of normal. Instead of having an increase in blood pressure during exercise, my blood pressure drops. My heart rate either doesn't go up on it's own, or goes up really high and then tries to pause mid-exercise. Fortunately, now I have a pacemaker that can help me compensate for some of my heart abnormalities, but when it's not programmed correctly (which has happened many many times) it actually hinders me more than helps.

Over the past several months I've had a lot of scary instances with pacemaker-mediated problems. Most of this centered around a switch from CLS mode which was no longer working to warn me of low BP or increase my rate appropriately during exercise, to DDDR mode which chooses a rate based on movement instead of autonomic tone. I had very serious problems in DDDR mode which landed me an overnight stay in the ER due to being paced to 180 in the ventricle every time I moved, and several forced evaluations inside ambulances by well-meaning bystanders.

I've reached out to Biotronik headquarters requesting help from a rep in a territory with a high volume of autonomic dysfunction patients: Toledo, where Dr. Grubb is. The reps that I've had have never had a patient with my condition before by their own admission. The number of Biotronik patients in general within my territory is extremely small. After several years of this, I feel the least that the company can do is provide a phone consult from a rep experienced with autonomic disorders or one who has worked with a serious runner before. So far they have not arranged this. My local rep (whom I've never met because he has never come to one of my appointments in 2 years) emailed me and said he was talking to someone in Toledo. Then he didn't show up to the last appointment once again and sent a list of settings to someone else completely unfamiliar to my case. We decided instead to try a set of parameters that I brought in from Dr. Grubb's study. I'm highly dissappointed with the service I've gotten from Biotronik. The ironic promotional tag they use: #biotronikcares, is a total joke.

My doctor on the other hand, has been very helpful, and I'm almost never able to say that about a mainstream MD. He spent 6 hours at the hospital trying to fix the pacing problems himself. That's unheard of. He arranged an implanted port for me so that I can get myself out of emergency hypotension and avoid the stress of constantly having to fight with the ER. I am incredibly grateful for the port. He will probably never know how grateful, because I don't think I will ever be able to fully express to him the desperation I was feeling before the port. I would get so sick that I couldn't stand, eat, drink or keep down BP and heart medications........frequently. My BP either wouldn't register or would run in the 60's/30's range. After days of this when I'd finally admit I needed help and tried to get it from the ER or urgent care, they did absolutely nothing for me and wouldn't even call either him (my main autonomic specialist) or the autonomic neurologist that also objectively confirmed the seriousness of my condition through testing. Trying to imagine the rest of my life being like that was unbearable. I don't have the strength to fight for a bag of salt water when I'm already so sick. If this is lifelong, I need a lifelong way to manage it......myself. Going to the ER every time I need BP control help would be the equivalent of a diabetic going to the ER every time their blood sugar is out of range. I already struggle with feeling like a failure in many ways due to this illness. Having a port and a way to help myself with symptoms gives me back some sense of independence.

In the middle of this summer, another unexpected problem occurred. While I was out running (something I've attempted every single day for 5 years to maintain conditioning and increase my energy levels) my hip started to hurt. I got to the end and sat down to stretch it out. That was the last time I was able to stand on it for several months. The pain became excruciating and it was later confirmed that I had fractured my femur. I'm in week 10 of a 16+ week running ban. Losing running was a huge huge blow to me. It was literally the only thing I had left of my pre-illness self that I was good at. It's my anti-anxiety and depression treatment. It boosts my BP and gives me a 90 minute break from hypotension every day post-run. I was devastated and I'm not even sure how I've managed to avoid sinking into an immense depressive state. To top it off, I had no physical therapy advice except to "try a bike" via email. When I told my orthopedist after 8 weeks of trying as hard as I possibly could (15-30 miles a day) that the bike was too difficult for me due to plummeting BP, he responded that I should just imagine I don't have that problem. That had me in tears outside. Using the diabetic analogy again since that disorder is well understood, would this orthopedist tell a type one diabetic to imagine they didn't have it if they expressed to him that a bike exacerbated their low blood sugar and they were collapsing and having 911 called on them (like I was)?

I don't think that some doctors realize the impact that their comments have on patients that are already silently struggling to keep it together physically and emotionally. Chronic illness takes a tremendous toll on a person. It is really tough to get up each day and try so hard knowing that no amount of "trying" is going to cure my situation. Each day will be basically the same and hopefully not worse than the last. Each day will be a struggle. "Imagining" that I don't have my condition and living in denial will culminate in collapsing because I ignored my hypotension warnings. Ironically, I'll then end up in the ER where I'll be blamed for the "imagining" (attempts to live life like a normal person) that got me into the situation.

I think that some of the doctors I've encountered are the ones that are in need of some imagining. I urge all medical professionals to truly try to imagine life from your patient's perspective and realize that what you say has a tremendous impact on your patients even if they don't openly express this to you. Try to imagine what your life would be like if tomorrow you were struck with the same set of symptoms that your patients are experiencing, how you would cope with that situation, and what you would want your doctors to say (and not say) to you!